Rabbit anti-Human UBA5 Polyclonal Antibody

Uniprot Id

Q9GZZ9

Target Species

Human

Target Name

UBA5

Target Full Name

Ubiquitin-like modifier-activating enzyme 5

Target Function

E1-like enzyme which specifically catalyzes the first step in ufmylation. Activates UFM1 by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a UFM1-E1 thioester and free AMP. Activates UFM1 via a trans-binding mechanism, in which UFM1 interacts with distinct sites in both subunits of the UBA5 homodimer. Trans-binding also promotes stabilization of the UBA5 homodimer, and enhances ATP-binding. Transfer of UFM1 from UBA5 to the E2-like enzyme UFC1 also takes place using a trans mechanism. Ufmylation is involved in reticulophagy (also called ER-phagy) induced in response to endoplasmic reticulum stress. Ufmylation is essential for erythroid differentiation of both megakaryocytes and erythrocytes.

Target Involvement

Epileptic encephalopathy, early infantile, 44 (EIEE44); Spinocerebellar ataxia, autosomal recessive, 24 (SCAR24)

Target Subcellular Location

Cytoplasm. Nucleus. Endoplasmic reticulum membrane. Golgi apparatus.

Target Protein Families

Ubiquitin-activating E1 family, UBA5 subfamily

Target Tissue Specificity

Widely expressed.

Target Synonyms

5730525G14Rik; AW240750; FLJ23251; FLJ23251UBA5; MGC105565; ThiFP1; UBA5; UBA5 ubiquitin activating enzyme E1 homolog; UBA5_HUMAN; UBE1DC; UBE1DC1; Ubiquitin activating enzyme 5; Ubiquitin activating enzyme E1 domain containing 1; Ubiquitin activating enzyme E1 domain containing protein 1; Ubiquitin like modifier activating enzyme 5; Ubiquitin-activating enzyme 5; Ubiquitin-activating enzyme E1 domain-containing protein 1; Ubiquitin-like modifier-activating enzyme 5; UFM1 activating enzyme; UFM1-activating enzyme

Target Background

This gene encodes a member of the E1-like ubiquitin-activating enzyme family. This protein activates ubiquitin-fold modifier 1, a ubiquitin-like post-translational modifier protein, via the formation of a high-energy thioester bond. Alternative splicing results in multiple transcript variants. A pseudogene of this gene has been identified on chromosome 1.